Well, we have had a few months now of calm, smooth sailing with Ian’s medical concerns. He has been stable and thriving... gaining weight, smiling, and advancing. So, naturally, it’s time to dive back in to something else... both old and new. Ian never keeps us bored for long and quickly comes up with new adventures for us.
(Vomiting and Feeding, Genetics & Metabolism, Diarrhea, Endocrine, Ear Tubes, and Pulmonary)
VOMITING and FEEDING
Within the last 3 weeks or so however, things have been changing. It started with vomiting. Since Ian has the Nissen fundoplication, he is unable to vomit, burp, spit-up, etc. However; after retching for so long after the surgery due to post-prandial hypoglycemia/ reactive hypoglycemia, he has loosened and herniated the surgical wrap. This was confirmed back in January with an Upper GI series at CHOP. Eventually, this will need repairing. Since the discovery of this, Ian’s vomiting has been slowly picking up speed. Now, he is vomiting about 5-6 times a week, sometimes a couple in one day, sometimes 2 days with nothing. It is very different from before though. He struggles to get it through and has a huge burp of air passage before the vomit follows. Due to the struggle to push it up, it falls out of his mouth. It is not forceful as before, but only because the Nissen wrap slows it down.
This has changed Ian’s feeding desires considerably. Back when he was hospitalized in January-February for the viral gastroenteritis bug, he was deprived of food and wanted nothing but to down hundreds of Cheerios and Goldfish crackers. Now, he is back to gagging and retching at the sight of, and even the mere mention of food words like “pretzel” and “fishy.”
Feeding therapy has been on hiatus clinically as well. We continue to do work at home, but with Ian’s lack of hunger, due to continuous feedings to keep his blood sugar stable from the reactive hypoglycemia, he has no desire to eat, or even play with or have exposure to food. This is disheartening because we were making some progress. I have fears that the vomiting is going to rear it’s ugly head all over again and we will be forced to replay the same horrible record, or try a different tune to dance along with. We will visit with CHOP surgical department (the group that monitors the hypoglycemia and control the feeding recipe and routine) on April 18th. I am concerned that a hernia repair and movement to the Toupet wrap is in the near future. I have discussed the difference in these in a previous BLOG:
I have hopes that we can begin to move Ian to a more normal feeding routine and slowly move away from the G tube and continuous meals. However, there are several factors that might get in our way: GI troubles in the intestines, endocrine diseases, and the like...... all of which will be covered momentarily...
At the request of the CHOP Genetics and Metabolic Diseases doctor today (we will discuss this appointment momentarily as well), we will be looking to “up the ante” on his feeding therapy soon. He is concerned that the longer we wait to push oral feeding with Ian, the harder it is going to be for him to re-learn to eat, chew, and sallow. Apparently, eating and swallowing is not like riding a bike. It needs to be taught, and then relearned as skills are unused. If anyone happens to know good feeding therapy techniques and/or strategies, please let me know.
GENETICS and METABOLISM
Today’s visit with CHOP was with a doctor in the Genetics and Metabolism department. This was uncharted territory for Ian. They ran some tests:
Some of these were run: http://www.chop.edu/service/biochemical-genetics-metabolic-disease/metabolic-testing-laboratory.html ...
1) CDT for Congenital Defects
2) Organic Acid Quantitation
3) Comprehensive Metabolic Panel
4) Amino Acid Quantitation, Plasma: Acylcarnitine Profile and Carnitine Profile
5) Cytogenetics Lab: Genomic Microarray Analysis with genome Wide Array
Most of these tests were run due to my severe HELLP syndrome during pregnancy and Ian’s need for immediate delivery at 29 weeks. Apparently, some mothers are carriers for certain fatty-acid genetics diseases that can then predispose them to HELLP syndrome and complications from it. There is some chance that contributed or caused my HELLP syndrome. If it played a part, there is a chance Ian is now expressive the trait as DOMINANT instead of my RECESSIVE carrier gene. This could lead to the problems he is experiencing.
Aside from my HELLP in the history, the doctor was not concerned about metabolic or genetics defects and diseases. Most children with these types of medical concerns experience developmental and motor delays, which Ian is not. However, he felt i was important to rule these concerns out of the way. The chromosomal testing will simply look for additions and deletions in the chromosomes. It will NOT look at mutations and “mapping” of each chromosome to see if there are defects. Most of the tests take one week to return. The chromosomal testing will take about 6 weeks.
To collect the urine, they place a baggie over the penis, or vulva of the child. It has an adhesive mesh that wraps around the entire opening of the bag and sticks to the child’s skin. This is great in theory, but unless the urine has helped to loosen the adhesive while peeing, this was NOT well thought out. Ian needed two bags, as he sweated half the first one off while waiting to pee, and both came off with much distress. Peeling adhesive off of a 19 month olds scrotum was NOT on my list of things to do today!!! :/ I guess I am thankful that he was not an adolescence with cognitive delays..... I can only imagine trying to use one of these after puberty has set in! Anywho... after waiting 3 hours, yes 3 HOURS for Ian to pee, he finally gave a sample and we could return home. I think he was rebelling against the adhesive mesh surrounding his package!!
At least while we were waiting we had the chance to meet several great families also doing outpatient lab work for their children. Ian enjoyed playing with the other children, urine bag and all!
The doctors main concern however, was meeting with GI and Endocrine for the eating, digestion, hypoglycemia issues....... which leads us to our next doctors appointments.
An interesting link... http://www.chw.edu.au/prof/services/biogen/biogen05.htm
On April 18th, once we meet with the surgeon about the hernia and feeding routines, we will meet with the GI department. Ian’s chronic diarrhea has not let up anymore-so than before. Ever since the hospital discharge from THE BUG (http://adventuresofbeaner.blogspot.com/2012/01/bug.html) and (http://adventuresofbeaner.blogspot.com/2012/02/bug-returns.html), he has continued to have 3-4 bloody and mucus filled poopy diapers daily. It is not from the food he is on because he was on it for over a month before the virus and all this started. There have been 5 c.diff toxin tests all come back negative and 3 negative stool cultures. This is not caused by a virus or bacteria. It may have ben triggered by one. Some diseases can be triggered by these types of things, such as Celiac Disease.
I am hoping that they will order a lower GI series or a Colonoscopy or Sigmoidoscopy to VIEW what is going on. I am tried of all the TESTS coming in negative. This clearly seems to be inflammatory related. It could also be a milk or food allergy which may need an Endoscopy. Wither way, I believe they need to go in and look. This would mean another hospital admission due to anesthesia. So, what else is new! :(
I would like to think that GI would see the longevity of this issue and act promptly to get it under control. We have been using a homemade diaper rash cream with: Aveeno diaper cream for the oatmeal, Rx Nystatin to help keep away fungus, Rx Triamcinolone to help with the inflammation, and Ilex cream to from a strong moisture barrier. I add some A&D ointment as well for barrier protection. I make this weekly and we are suing it every time. It seems to be the only way to treat and AVOID the diaper rash that ends up bleeding from it’s severity. The diarrhea has completely changed his lower GI patterns and care.
Personally, my mind is set on Inflammatory Bowel Disease, Celiac’s Disease, or Milk Protein Allergy....... but then again, I was set on c.diff too! Hopefully testing will show something soon.
Also, the genetics and Metabolism doctor wanted us to see GI again to make sure the GERD and Nissen were being managed. The surgeon has been helping us tremendously with that. The great thing about seeing a surgeon as an overall caregiver, is they have an enormous amount of training in SO MANY areas that they are like having tons of specialists all rolled into one person! Fabulous!!!
However, there does seem to be a new concern after today to visit with Endrocrinology for diseases in that department. Due to Ian’s hypoglycemia issues, even the post-prandial kind, other diseases should be considered and hopefully ruled out. The main concern is Hyperinsulism:
Fortunately, CHOP is the #1 hospital for Hyperinsulinism in the country. I have friends who have visited this clinic with their children and have had wonderful care and knowledge there. One doctor in particular is highly coveted and we will be trying to meet with him within the next couple months...... hopefully sooner :(
Endocrinology is something that has always been floating around in Ian’s care, but never in the forefront. Neonatologists at the NICU mentioned visiting them down the road for growth and hormone issues if stature was a concern. I did not know it would be for something like this. It would explain a lot, but I am hoping that the reactive hypoglycemia diagnosis takes care of his issues. On the flip side, having a a diagnosis, or a “non-diagnosis” is helpful either way.
Just as a reminder, when Ian eats a meal or any ind, oral or G tube, he “dumps” from his stomach later on..... releasing a large amount of glucose (sugar) into his blood stream. This then triggers a large release of insulin...... too much insulin. What happens is that the insulin “takes up” the glucose into the liver to be stored as glycogen (future energy storage for blood sugar buffering). With Ian, since there is so much insulin released, he runs out of glucose before insulin, causing very low blood sugars which lead to retching, vomiting, paleness, sweating, nausea...... all of which he was experiencing post-Nissen surgery before visiting the surgeon at CHOP for feeding maintenance.
If this is not what is entirely happening, it could be associated with Hyperinsulinism. There are certain “fasts” and sugar lab work and medication testing that can be done to help diagnosis and treat this condition if need be.
EAR TUBES and RESPIRATORY
Completely separate issue- or is it... Ian’s surgery for his ear tubes is on April 26th. Right now, Ian has had chronic fluid build-up behind his ear drums for over 6-9 months now.
- Since he is suffering from this and it could lead to “glue-ear,” along with the fact that
- He cannot tolerate antibiotics to treat ear infections (due to his Gi sensitivities) and also
- His recent history of recurrent ear infections, we will be doing the tubes.
It will still be done as outpatient, but because Ian is “eventful” on paper, the anesthesiologist wants to do it at the hospital and not an outpatient center. This put the surgery farther out, but provides much more safety for Ian.
I know sometimes, tubes need replacing. Over time, your body “grows” and the ear drums shed just as skin does.... with this the tubes are shed. Since this is in an “older child” the need for the tubes no longer exists because the anatomy has changed to allow fluid to not cause issues. In some children, and adults, however, this is not the case and tubes must continually be replaced. I hate to sound pessimistic, but knowing my child....... I’m counting on this being him! Hoping not, but we will see.
This could be contributing to his sinus and respiratory complications that occur on-and-off as well. Ian has had sinus issues ever since the sinusitis event back in December that was controlled......... and then the Nasonex led to the Nosebleed event..... which I would not like to re-live at this moment!! Right now, the chronic sinusitis condition is managed by Xlear nasal spray and was mostly eliminate by the Omnicef antibiotic he took previously.
So, as it looks right now, Ian’s April calendar is as follows:
***Today, 4th: CHOP genetics and Metabolism testing
***April 6th: Loudoun County VA infants and toddlers assessment done annually for OT, PT, and Speech services, etc.
***April 18th: CHOP GI and Surgical feeding appointments
***April 26th: Ear Tube surgery here in VA
***Somewhere in there we followup with the Pulmonolgist again for the sinusitis and GERD management
***And also, we are trying to get in with CHOP Endocrinology ASAP
***And to top it off, after visiting with GI and Surgery for feeding management, we most likely will need admittance for lower GI studies and potentially another surgery for Nissen hernia Repair.
Stay tuned as I will keep everyone posted as the information comes to us.
On a non-medical note....... we have finally moved and have downsized by more than half. It has been a challenge so far, but we are enjoying the lower maintenance and the opportunities it presents to us and Ian. He is enjoying the stairs, as usual, and somehow, miraculously remains outwardly as a happy toddler going about his days as if unfazed by anything. He has the sweetest little “ha-ha-ha” laugh and it makes us smile every time. He has also cut another tooth, letter “S,” the lower right molar.
It was a fussy one to come in and troubled in for days. Thankfully, it is erupted through the gums and can come in........ until another one comes along and does the same thing! ;)
Thank you again for all of your thoughts, prayers, love, and support during our journey! It means so much to our family to have the love of so many, near and far in physicality, but never far from our hearts!